PEDIATRIC/CRANIOFACIAL Adopted Children with Cleft Lip and/or Palate: A Unique and Growing Population Jordan W. Swanson, M.D., M.Sc. James M. Smartt, Jr., M.D. Babette S. Saltzman, Ph.D. Craig B. Birgfeld, M.D. Richard A. Hopper, M.D., M.S. Joseph S. Gruss, M.D. Raymond Tse, M.D. Seattle, Wash.; and Dallas, Texas
Background: Standard clinical pathways are well established for children with cleft lip and/or palate. Treatment of internationally adopted children differs because of the late age at presentation, a newly evolving child-family relationship, and variable extent and quality of previous treatment. Methods: The authors characterized the presentation and treatment patterns of all internationally adopted children with clefts at their institution between 1997 and 2011. Results: Among 1841 children with clefts, 216 (12 percent) were internationally adopted: 78 percent had cleft lip and palate, 18 percent had cleft lip, and 4 percent had cleft palate. Patients originated predominantly from China (80 percent). Median age at presentation was 31 months, and the rate of new presentations increased five-fold during the study period. Eighty-two percent presented with prior cheiloplasty, and revision was recommended for 64 percent of them. Thirty-seven percent of patients had prior palatoplasty, of which 34 percent presented with a palatal fistula. Secondary palatoplasty/pharyngoplasty was performed more frequently for patients who underwent primary palatoplasty before adoption than after adoption (95 percent CI, 0.20 to 0.77). Overall, adoptees required secondary surgery more often than nonadoptees (49 percent versus 28 percent) regardless of where their primary surgery was performed. Changes in adoptee growth indices suggested improvements in systemic health following adoption. Conclusions: Internationally adopted children with clefts have unique treatment challenges. Children with unrepaired clefts undergo surgery late, and children with prior repairs frequently undergo revision. Compared with nonadoptees, adoptees require more revisions and have a higher fistula rate. Further detailed study is important to optimize care. (Plast. Reconstr. Surg. 134: 283e, 2014.)
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hildren with cleft lip and/or palate who have been adopted from outside of the United States are presenting to our institution with increasing frequency. Although recent social and political changes have decreased total worldwide adoptions, patients with cleft deformities and other medical needs continue to be prioritized From the Craniofacial Center, Seattle Children’s Hospital; the Division of Plastic Surgery, Department of Surgery, University of Washington; and the Department of Plastic Surgery, University of Texas Southwestern. Received for publication November 7, 2013; accepted January 23, 2014. Presented at the 51st Annual Meeting of the Northwest Society of Plastic Surgeons, in Maui, Hawaii, February 16 through 20, 2013; and the 12th International Congress on Cleft Lip/Palate and Related Craniofacial Anomalies, in Orlando, Florida, May 5 through 10, 2013. Copyright © 2014 by the American Society of Plastic Surgeons DOI: 10.1097/PRS.0000000000000391
for adoption.1–3 The care of adoptees with clefts differs from that of children who have been managed along well-established clinical pathways that begin before birth: age of initial presentation to our center is older; previous social or physical trauma is common4; the extent and quality of previous treatment is variable; and the child-family relationship is new and evolving. We know little about how best to treat this unique group. There is scant literature on the patterns of presentation or timeline of treatment among these patients, or how they grow and develop through childhood. To date, the only published report comes from Sweden, where Hansson et al. described 25 internationally adopted patients Disclosure: The authors have no financial interest to declare in relation to the content of this article. No funding was secured for this study.
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Plastic and Reconstructive Surgery • August 2014 with cleft deformities, and noted technical and social challenges to their care.5 The goal of this study was to determine the characteristics at presentation, treatment patterns, revision rates, and factors affecting revision rates among internationally adopted patients with cleft lip and/or palate.
PATIENTS AND METHODS This study was approved by our institutional review board (Seattle Children’s Hospital, no. 13968). We performed a retrospective cohort study including all children with cleft lip and/ or palate presenting to the Craniofacial Center at Seattle Children’s Hospital between 1997 and 2011. Medical records for all patients were reviewed; information pertaining to demographic factors, characteristics at presentation, and treatment (both before and after adoption) were abstracted according to a predefined rubric. We included patients born outside of the United States and Canada who were legally adopted and lived within the United States at the time of initiation of care at our center. Patients for whom adoption status was unclear and those adopted within the United States through foster care were excluded. Cleft type was determined by physical examination performed on presentation by the craniofacial pediatrician and pediatric plastic surgeon. We designated isolated cleft lip with or without cleft alveolus; cleft lip and palate; and isolated cleft palate. Cleft lip with or without cleft alveolus
and cleft lip and palate were further subclassified as unilateral or bilateral. For comparison, we abstracted information about and calculated the timing of surgical procedures and revision rates for nonadopted patients. Care at Seattle Children’s Hospital was provided in accordance with team guidelines outlined by the American Cleft Palate Association. Specialists in pediatrics, nursing, plastic surgery, otolaryngology, audiology, speech pathology, dentistry, and oral surgery provide multidisciplinary care. Records from all specialities were reviewed. Study data were collected and managed using REDCap (Research Electronic Data Capture) hosted at the University of Washington and analyses were performed using Stata version 12.1 (StataCorp, College Station, Texas).6 Univariate analysis of presentation characteristics and revision rates was performed using chi-square testing.
RESULTS Demographics Among 1841 children with cleft lip and/ or palate, 216 (12 percent) were internationally adopted. New presentations of international adoptees increased from 5 per year, to more than 25 per year from 2005 to 2010, an average five-fold increase during the study period (Fig. 1). The most common countries of origin included China (80 percent), Russia (5 percent),
Fig. 1. Incidence of internationally adopted children with cleft lip and/or palate. Consecutive patients presenting to our center from 1997 to 2011 were screened. The number of new internationally adopted patients has increased five-fold during the past 15 years. CP, isolated cleft palate; CL, cleft lip with or without cleft alveolus; CLP, cleft lip and palate.
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Volume 134, Number 2 • Adopted Children with Cleft Lip/Palate and Thailand (3 percent) (Fig. 2). Compared with U.S. adoption patterns over the same period, our population had a greater proportion of Chinese and smaller proportions of Russian and Korean adoptees.1 The male-to-female ratio was 1:1 for children with cleft lip with or without palate and 1:8 for children with isolated cleft palate. Seventy-eight percent of children had cleft lip and palate, 18 percent had isolated cleft lip with or without cleft alveolus, and 4 percent had isolated cleft palate (Fig. 3). The distribution of clefts by type remained consistent over the study period, with cleft lip and palate deformities representing greater than 50 percent of new presentations every year. The median age at adoption was 2.0 years. Most patients were first evaluated at our institution within 2 months of adoption (Fig. 4), unless they were initially cared for by another U.S. cleft team following adoption; 27 patients (13 percent) received prior surgical care at another U.S. institution before transferring to ours (data not shown). The average age at adoption remained fairly constant during the study period. Care in Country of Origin Most children with cleft lip with or without cleft alveolus had preadoption cheiloplasty (77 percent) (Fig. 5). Median age at cheiloplasty was
Fig. 3. Cleft type of international adoptees. UCL, unilateral cleft lip with or without cleft alveolus; BCL, bilateral cleft lip with or without cleft alveolus; UCLP, unilateral cleft lip and palate; BCLP, bilateral cleft lip and palate; CP, isolated cleft palate.
0.7 ± 0.6 year for unilateral cleft lip with or without cleft alveolus and 2.1 ± 1.0 years for bilateral cleft lip with or without cleft alveolus. Most children with cleft lip and palate also had preadoption cheiloplasty (85 percent); however, a minority had undergone preadoption palatoplasty (37 percent). Among children with cleft lip and palate, preadoption cheiloplasty and palatoplasty were performed at respective median ages of 1.0 and 2.2 years for unilateral cleft lip and palate and 1.4 and 2.7 years for bilateral cleft lip
Fig. 2. Country of origin of adoptees with cleft lip and/or palate presenting to our center and of overall intercountry adoptees to the United States. There were fewer than two adoptees for several countries of origin in our study group (i.e., Bolivia, Guatemala, Liberia, Philippines, Romania, and Sierra Leone). Overall intercountry adoption statistics, in thousands, were obtained from the U.S. State Department (using 1999 to 2011 data, extrapolated to the 1997 to 2011 period using a multiplier of 1.15; from U.S. Department of State. 2012 Annual Report on Intercountry Adoption. Washington, DC: Office of Children’s Issues, Bureau of Consular Affairs; 2013). Countries of origin with more than one adoptee are displayed.
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Fig. 4. Patient age at adoption and initial presentation.
and palate. Similarly, 33 percent of children with isolated cleft palate had preadoption palatoplasty, at a median age of 2.0 ± 0.1 years. A decreasing proportion of patients are undergoing surgical repair before adoption (Fig. 6). Rates of preadoption cheiloplasty dropped from 100 percent to 62 percent, and rates of preadoption palatoplasty have dropped from 62 percent
to 18 percent (an average 10 percent reduction per year for each). Rates of preadoption repair differed by sex: 91 percent of boys versus 83 percent of girls presented with preadoption cheiloplasty (p = 0.04), and 39 percent of boys versus 33 percent of girls presented with preadoption palatoplasty (p = 0.03). The rates of repair differed by country of origin.
Fig. 5. Cheiloplasty and/or palatoplasty before adoption according to cleft type. CL, cleft lip with or without cleft alveolus; CLP, cleft lip and palate; CP, isolated cleft palate.
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Fig. 6. Trends in rates of cheiloplasty and/or palatoplasty before adoption (includes years with ≥15 adoptees).
However, given the few adoptees from most countries, little can be inferred from these data. Cleft Lip: Presentation and Treatment Thirteen percent of adoptees with cleft lip (cleft lip with or without cleft alveolus or cleft lip and palate) presented with no prior repair. Of the 87 percent with prior repairs, 82 percent were performed before adoption and 5 percent were performed after adoption at another institution before transfer to our center (Table 1). Residual stigmata were common, including orbicularis muscle discontinuity (38 percent), significant nasal asymmetry (69 percent), and alar base malposition (29 percent). Lip revision was recommended for 63 percent who underwent repair before adoption and 55 percent who had undergone repair in the United States before presentation. A complete revision was recommended in most cases. Approximately 85 percent of patients in each group for whom revision was recommended chose to undergo surgery at our institution. Among patients with bilateral deformities, revision was recommended (70 percent) and carried out (53 percent) more frequently than for patients with unilateral deformities (57 percent and 42 percent, respectively). Either major or minor revision was performed in 36 percent of patients who underwent cheiloplasty at our institution, and was also more common for bilateral deformities. No association between country of
origin, side(s) of cleft, palatal involvement, sex, age at adoption, age at repair, or repair type and recommendation for revision was found. Cleft Palate: Presentation and Treatment Half (49 percent) of the adoptees with cleft palate (cleft lip and palate or isolated cleft palate) presented with no prior repair. Of the 51 percent with prior palatoplasty, 37 percent had preadoption repair and 14 percent had postadoption repair at another institution before transfer to our center (Table 2). Velopharyngeal dysfunction was noted at presentation in 48 percent of adoptees with preadoption palatoplasty and in 36 percent of adoptees with postadoption palatoplasty at another U.S. institution. Palatal fistulas were present in approximately 33 percent of patients with prior repair, either before adoption or after adoption but at another U.S. institution. Fourteen percent of patients whose primary palatoplasty was carried out at our institution developed a fistula; relative to the preadoption repair group, these patients were 70 percent less likely to develop a fistula (OR, 0.31; 95 percent CI, 0.14 to 0.69). Relative to patients with preadoption palatoplasty, palatal fistula repair was performed more frequently for patients whose palatoplasty was performed at another U.S. institution (OR, 1.39; 95 percent CI, 0.48 to 3.99) but less frequently for patients whose palatoplasty was performed at our center (OR, 0.58; 95 percent CI, 0.25 to 1.36).
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Plastic and Reconstructive Surgery • August 2014 Table 1. Cleft Lip Presentation and Treatment Patterns among Internationally Adopted Children with Cleft Lip and/or Palate Presenting to the Seattle Children’s Hospital Craniofacial Center between 1997 and 2011 Prior Repair Primary Cheiloplasty Cleft type Unilateral Bilateral Median age at cheiloplasty, yr Median age at adoption, yr Median age at presentation, yr Prior cheiloplasty at presentation Unilateral repair type Rotation advancement* Inferior triangle† Straight-line repair‡ Other Bilateral repair type Prolabial vermillion retained§ Lateral vermillion advancement║ Other Unknown Stigmata on presentation Lip length discrepancy Orbicularis discontinuity Nasal deformity Nasolabial/orolabial fistula Palatal fistula White roll discontinuity Vermillion defect/deficiency Alar base malposition Orbicularis tethered to alar base Heavy scarring Wide philtrum/prolabium Revision recommended at presentation Complete revision Minor revision¶ Patients underwent lip revision Mean age at lip revision, yr
Unrepaired
Preadoption (n = 170) (%)
Other U.S. (n = 11) (%)
SCH (n = 26)
123 (72) 47 (28) 1.1 ± 1.0 3.1 ± 2.4 3.3 ± 3.6
4 (36) 7 (64) 1.2 ± 0.5 1.2 ± 0.8 7.3 ± 6.1
22 (85) 4 (15) 1.8 ± 0.8 1.5 ± 0.7 1.6 ± 0.8
33 (59) 13 (23) 4 (7) 6 (11)
1 (50) 1 (50) 0 0
n/a n/a n/a n/a
4 (16) 19 (76) 2 (8) 95
1 (33) 2 (67) 0 6
n/a n/a n/a n/a
45 (26) 64 (38) 118 (69) 54 (32) 19 (11) 30 (18) 46 (27) 49 (29) 22 (13) 20 (12) 7 (4) 108 (64) 85 (50) 23 (14) 88 (52) 5.7 ± 3.2
2 (18) 4 (36) 8 (73) 5 (46) 2 (18) 4 (36) 5 (46) 5 (46) 2 (18) 2 (18) 2 (18) 6 (55) 4 (36) 2 (18) 5 (46) 11.5 ± 7.1
n/a n/a n/a n/a n/a n/a n/a n/a n/a n/a n/a n/a n/a n/a 9 (35) 5.7 ± 3.4
n/a, not applicable. *Millard, Mohler, and Noordhoff techniques. †Tennison-Randall techniques. ‡Nakajima and Pfeiffer techniques. §Manchester technique. ║Millard and Mulliken techniques. ¶Includes local skin flap or Z-plasty, or correction of white roll, vermillion, or mucosa.
Surgery for velopharyngeal dysfunction (e.g., secondary palatal lengthening, sphincter pharyngoplasty) was performed less often for those who underwent palatoplasty at other U.S. hospitals (OR, 0.79; 95 percent CI, 0.31 to 2.04) and at our center (OR, 0.39; 95 percent CI, 0.20 to 0.77) relative to those who underwent palatoplasty before adoption. The mean age at palatoplasty for adoptees treated at our center was the same as for those adoptees who had preadoption repair (2.4 years). Patterns among Adopted and Nonadopted Children During the study period, 1808 patients with cleft diagnoses underwent surgery at our center. Adopted children represented 10 percent of 399 patients with cleft lip with or without cleft alveolus,
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25 percent of 660 patients with cleft lip and palate, and 2 percent of 533 patients with isolated cleft palate. The overall revision rate for adoptees was higher (49 percent) than for nonadoptees (28 percent), and this was true regardless of whether they had previously undergone primary cheiloplasty/palatoplasty at our center or at another institution (Fig. 7). Given these differences, we performed a detailed review of nonadoptees who underwent palatoplasty at our institution in 2007 (the peak and median year for adoptees) to serve as a comparison group. Nonadoptees had lower fistula and revision rates than adoptees that underwent palatoplasty at our institution or at another institution (Table 2). Mean age at both primary cheiloplasty and palatoplasty was more than 1 year older for
Volume 134, Number 2 • Adopted Children with Cleft Lip/Palate Table 2. Cleft Palate Presentation and Treatment Patterns among Internationally Adopted Children with Cleft Lip and/or Palate Presenting to the Seattle Children’s Hospital Craniofacial Center between 1997 and 2011 Adoptees Unrepaired
Preadoption (n = 64) (%)
Other U.S. (n = 25) (%)
SCH (n = 86) (%)
SCH (n = 45) (%)
61 (95) 3 (5) 4.2 ± 2.6 2.4 ± 2.0 4.6 ± 2.6
25 (100) 0 (0) 1.7 ± 0.9 2.0 ± 0.9 7.0 ± 4.8
80 (93) 6 (7) 1.9 ± 1.1 2.4 ± 1.1 2.1 ± 1.1
20 (44) 25 (56) n/a 1.0 ± 0.1 n/a
31 (48) 22 (34) 33 (52) 14 (22)
9 (36) 8 (32) 11 (44) 7 (28)
n/a 12 (14)* 25 (31)* 12 (15)
n/a 3 (7)* 9 (20)* 3 (7)
25 (39) 6 (9) 2 (3) 6.3 ± 3.2 4.4 ± 3.0
6 (24) 5 (20) 0 (0) 7.2 ± 4.8 5.2 ± 4.4
13 (16) 9 (11) 3 (4) 4.9 ± 2.4 4.6 ± 3.5
4 (9) 2 (4) 0 (0) 4.2 ± 1.3 5.5 ± 1.9
Prior Repair Primary Palatoplasty Cleft type CLP iCP Median age at adoption, yr Median age at palate repair, yr Median age at presentation, yr Presentation characteristics║ Velopharyngeal insufficiency Palatal fistula† Secondary palatal surgery‡ Palatal fistula repair§ Other palatal revision surgery Furlow/secondary palatoplasty Sphincter pharyngoplasty Other Mean age at secondary palatal surgery, yr Mean follow-up from initial presentation, yr
Nonadoptees Palatoplasty in 2009
CLP, cleft lip and palate; iCP, isolated cleft palate; SCH, Seattle Children’s Hospital; n/a, not applicable. *p< 0.05 relative to the rate for those with preadoption repair. †OR, 0.90 (95% CI, 0.34 to 2.41) for other U.S. hospitals; OR, 0.31 (0.14 to 0.69) for SCH; and OR, 0.14 (95% CI, 0.04 to 0.49) for nonadoptees. ‡OR, 0.74 (95% CI, 0.29 to 1.87) for other U.S. hospitals; OR, 0.39 (95% CI, 0.20 to 0.76) for SCH; and OR, 0.23 (95% CI, 0.10 to 0.57) for nonadoptees. §OR, 1.39 (95% CI, 0.48 to 3.99) for other U.S. hospitals; OR, 0.58 (95% CI, 0.25 to 1.36) for SCH; and OR, 0.49 (95% CI, 0.13 to 1.87) for nonadoptees. ║For initially unrepaired patients, palatal fistulas are reported for the postoperative study period.
adoptees (Fig. 8). Age at palate revision also differed (mean age, 5.9 years for adoptees versus 2.9 years for nonadoptees). However, the age difference was negligible for alveolar bone grafting. Growth Patterns Age-expected percentiles for head circumference, height, and weight at presentation and at last follow-up are illustrated in Figure 9. Among adoptees who had undergone postadoption palatoplasty at another U.S. institution, there was little to no change. Adoptees who had undergone preadoption repair and presented immediately after adoption showed moderate improvements in growth measures (8, 11, and 8 percent change for head circumference, height, and weight, respectively). In adoptees who presented with unrepaired cleft palate and underwent postadoption palatoplasty at our center, the initial height and weight were lowest and improvements in all growth indices were the greatest (12, 13, and 19 percent for head circumference, height, and weight, respectively). Speech, Dental, and Otolaryngologic Patterns On initial speech and language pathologic assessment, 50 children (23 percent) were diagnosed with suspected velopharyngeal dysfunction.
Fifteen patients (7 percent) presented with articulation errors, and 36 patients (16 percent) presented with language acquisition delay. One hundred fifty-one children (70 percent) received an initial dental assessment. Of these, 39 (18 percent) had dental caries and 44 (20 percent) had a collapsed arch with crossbite. All patients with cleft palate were assessed by an otolaryngologist; 46 (26 percent) had a history of ear infections, 12 (7 percent) had had previous tympanostomy tubes placed, and 43 (25 percent) were found to have conductive hearing loss. Myringotomy tubes were indicated for 114 presenting patients (65 percent) with palatal clefting; this proportion was higher in the later years of the study period.
DISCUSSION Care of adoptees with cleft lip and/or palate differs from that of nonadopted children who have been managed since infancy. International adoptees arrive to a new country, with a new language and a new family, but also have surgical needs that have not, or only partially, been met. Despite major ongoing changes to global adoption patterns, we anticipate that the volume of adoptions
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Fig. 7. Rates of secondary surgery for adoptees and nonadoptees. SCH, Seattle Children’s Hospital.
of children with medical needs, including patients with clefts, will remain relatively stable. Presentation Our craniofacial center experienced a focal expansion of internationally adopted children with clefts approximately 10 years ago, and this population constitutes approximately 15 percent of our current cleft patient volume. The majority of adoptees at our institution are from China, and this may be because of Seattle’s location on the Pacific Rim.
Because the distribution of cleft types may vary by ethnicity, we compared our predominantly Chinese patients to a recently published distribution of cleft anomalies in China.7 A higher ratio of children with cleft lip and palate than with isolated cleft palate presented to our center than would be expected. This may reflect greater willingness to give up children with greater medical needs to families in resource-rich countries through adoption. The low incidence of adoptees with isolated cleft palate may reflect lower detection, higher
Fig. 8. Age at surgery for adoptees and nonadoptees. CL, cleft lip with or without cleft alveolus; CLP, cleft lip and palate; CP, isolated cleft palate.
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Fig. 9. Head circumference, height, and weight (as mean age-expected percentiles) among adoptees on presentation and at last follow-up. Patients are grouped according to where their palatoplasty was performed. Patients who underwent palatoplasty at another U.S. institution presented on average 5 years after adoption. Patients who underwent palatoplasty before adoption or had unrepaired cleft palate presented soon after adoption (0.4 and 0.2 years) are included. SCH, Seattle Children’s Hospital.
mortality secondary to poor feeding, or lesser willingness to give up children who do not have external facial differences. We recorded a high incidence of female adoptees: for cleft lip with or without palate, the femaleto-male ratio was 1:1, compared with the expected ratio of 1:2. For isolated cleft palate, the femaleto-male ratio was 8:1, compared with the expected ratio of 2:1.7 This could reflect a higher likelihood of giving up a girl for adoption,8 and may increase for a more visible defect. We also found that boys were significantly more likely than girls to undergo cheiloplasty or palatoplasty before adoption. Although social scientists have identified gender-preferential distribution of resources in some of the countries from which our patients originate,9 we do not have sufficient insight into the reasons for this disparity. Surgical Care Most adoptees underwent cheiloplasty before adoption, but few presented with previous palatoplasty. The reasons for this may include the timing of the adoption process, an underappreciation of
the importance of palatoplasty, a greater stigma associated with a highly visible cleft lip with or without cleft alveolus, or reluctance to proceed with a procedure associated with greater risks. We noted that the rates of both cheiloplasty and palatoplasty before adoption decreased in spite of a fairly constant age of adoption during the study. The reasons for this trend are unclear, but we speculate that rising rates of international adoption have resulted in recognition that surgical care can be deferred to a receiving team in a resource-rich country. We found that the mean age of children without prior repairs was younger than of those with prior repairs; it may be that surgery in the country of origin is deferred if an international adoption is pending. The majority of adoptees presenting to our institution with preadoption cheiloplasty required revision (64 percent), and we found a high incidence of fistulas (33 percent) and suspected velopharyngeal dysfunction (48 percent) in adoptees who underwent preadoption palatoplasty. Although the quality of previous care was sometimes excellent, in some instances, previous
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Plastic and Reconstructive Surgery • August 2014 surgical scarring precluded successful correction of residual deformities, required extensive revision, or limited results that could be achieved at the next stage of treatment. The odds that an adoptee required secondary palatal surgery was significantly greater among children who had preadoption palatoplasty relative to those who underwent postadoption palatoplasty. Better nutritional status and perioperative care following adoption may improve wound healing. However, specific inferences regarding care in the country of origin cannot be made, given that adoptees come from multiple countries, each with varying clinical practices, providers, and adoption policies. Although consensus holds that palatoplasty by 12 months optimizes speech outcomes,10 it is unclear what the relative impact of delayed palatoplasty is at older ages (i.e., 2 years) or for children facing transition to a foreign language. When consulted in specific cases where an adoption is confirmed, we sometimes consider deferring surgical treatment until arrival if the resulting delay to surgery is minimal. This provides opportunities for continuity of surgical care and for a permanent family to support the adoptee as he or she undergoes surgery. Ultimately, in our study, the mean age of palatoplasty was the same whether it was performed before or after adoption (mean, 2.4 years). In the case of unrepaired cleft lip and palate, some surgeons argue that the palate should be repaired first, given that delay may impact speech development.11 However, there are no definitive data to support this view. Other surgeons suggest simultaneous cheiloplasty and palatoplasty; however, this is technically challenging and may involve greater risks.12–14 We generally start with cleft lip repair and perform cleft palate repair within 3 months. The relative impact of this delay for adoptees is unclear, and the relative merits of the various approaches deserve further study in this population. Compared with nonadoptees at our center, fistula and surgical revision rates are higher in adoptees regardless of whether their primary repair was performed at our institution or at another. Many factors may contribute to these less favorable results, including late age at initial repair, malnutrition, and ethnic variations. We found higher revision rates for adoptees that underwent primary repair at other U.S. institutions; however, this may be attributable to referral bias, as a substantial proportion of these children transferred care because of complications or previous unfavorable results.
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Growth/Nutrition and Interdisciplinary Care We looked at trends in growth indices as a way of inferring changes in nutritional status (Fig. 9 and Table 2). Adopted children who underwent palatoplasty at another U.S. institution presented to our center on average 5 years after adoption. As expected, we measured little change in their growth percentiles because their period of catchup growth took place before arriving to our center. In contrast, children who either had preadoption repair or unrepaired cleft palate presented to our center soon after adoption (average, 0.4 and 0.2 years after adoption, respectively). We found improvements in all growth percentiles for both groups, suggesting improvements in nutritional status.15,16 Given the impact of nutritional status on wound healing, consideration of a preoperative catch-up growth period may be warranted. Additional findings on presentation included high rates of dental and otologic disease. These underscore the importance of interdisciplinary expertise of craniofacial centers in the treatment of this population.17 Speech and Social Concerns Given the implications of late palatoplasty on speech, providers and families may have an urgency to proceed with surgery but may also be reluctant to do so given the child’s vulnerable circumstances following adoption. The surgical experience for families who have recently adopted a child deserves further study to identify the factors that might predict best outcomes (including speech) for this unique population.
CONCLUSIONS Internationally adopted children with cleft lip and/or palate constitute a substantial portion of cleft patients at our institution and likely in the United States. Most of these children have undergone cleft lip repair but only one-third have undergone palate repair. Treatment of these children varies greatly from our normal protocol, as the age at primary surgery is often delayed and the revision rate is high. Increases in growth percentiles following adoption suggest improvements in systemic status that may improve surgical success. The need for secondary surgery and the rate of palatal fistula were lower among adoptees who underwent primary repair at our center than among those who underwent repair before presentation. Regardless of where the primary repairs were performed, adoptees had a higher rate of fistulae and secondary surgery compared
Volume 134, Number 2 • Adopted Children with Cleft Lip/Palate with nonadoptees. Surgical revisions help adoptees to converge with their peers who have been treated in cleft centers since infancy, and their repair timeline converges by the time of alveolar bone grafting. Raymond Tse, M.D. Division of Craniofacial and Plastic Surgery Seattle Children’s Hospital M/S OB.9.527 4800 Sand Point Way NE Seattle, Wash. 98105 [email protected]
ACKNOWLEDGMENT
The authors thank Maren Shipe for her assistance in gathering further data following peer review. REFERENCES 1. U.S. Department of State. 2012 Annual Report on Intercountry Adoption. Washington, DC: Office of Children’s Issues, Bureau of Consular Affairs; 2013. 2. Webley K. The baby deficit. Time 2013;181:34–39. 3. Johnson DE. International adoption: What is fact, what is fiction, and what is the future? Pediatr Clin North Am. 2005;52:1221–1246, v. 4. Weitzman C, Albers L. Long-term developmental, behavioral, and attachment outcomes after international adoption. Pediatr Clin North Am. 2005;52:1395–1419, viii. 5. Hansson E, Svensson H, Becker M. Adopted children with cleft lip or palate, or both, require special needs cleft surgery. J Plast Surg Hand Surg. 2012;46:75–79.
6. Harris PA, Taylor R, Thielke R, Payne J, Gonzalez N, Conde JG. Research electronic data capture (REDCap): A metadatadriven methodology and workflow process for providing translational research informatics support. J Biomed Inform. 2009;42:377–381. 7. Meng T, Shi B, Zheng Q, Wang Y, Li S. Clinical and epidemiologic studies of nonsyndromic cleft lip and palate in China: Analysis of 4268 cases. Ann Plast Surg. 2006;57:264–269. 8. Hvistendahl M. Unnatural Selection: Choosing Boys over Girls, and the Consequences of a World Full of Men. New York: PublicAffairs; 2011. 9. Sen A. Missing women. BMJ 1992;304:587–588. 10. Peterson-Falzone SJ. The relationship between timing of cleft palate surgery and speech outcome: What have we learned, and where do we stand in the 1990s? Semin Orthod. 1996;2:185–191. 11. Sullivan S, Jung YS, Mulliken JB. Outcomes of delayed cleft palate repair for internationally adopted children. Paper presented at: 2013 International Congress on Cleft Lip/ Palate and Related Craniofacial Anomalies; May 9, 2013; Orlando, Fla. 12. Bardach J, Kelly KM, Salyer KE. A comparative study of facial growth following lip and palate repair performed in sequence and simultaneously: An experimental study in beagles. Plast Reconstr Surg. 1993;91:1008–1016. 13. Bardach J, Roberts DM, Yale R, Rosewall D, Mooney M. The influence of simultaneous cleft lip and palate repair on facial growth in rabbits. Cleft Palate J. 1980;17:309–318. 14. Morioka D, Yoshimoto S, Udagawa A, Ohkubo F, Yoshikawa A. Primary repair in adult patients with untreated cleft lipcleft palate. Plast Reconstr Surg. 2007;120:1981–1988. 15. Jenista JA. Special topics in international adoption. Pediatr Clin North Am. 2005;52:1479–1494, ix. 16. Jenista JA, Chapman D. Medical problems of foreign-born adopted children. Am J Dis Child. 1987;141:298–302. 17. Van Dyke DC, Canady JW. Management of the adopted child in the craniofacial clinic. J Craniofac Surg. 1995;6:143–146.
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Background: Standard clinical pathways are well established for children with cleft lip and/or palate. Treatment of internationally adopted children differs because of the late age at presentation, a newly evolving child-family relationship, and variable extent and quality of previous treatment. Methods: The authors characterized the presentation and treatment patterns of all internationally adopted children with clefts at their institution between 1997 and 2011. Results: Among 1841 children with clefts, 216 (12 percent) were internationally adopted: 78 percent had cleft lip and palate, 18 percent had cleft lip, and 4 percent had cleft palate. Patients originated predominantly from China (80 percent). Median age at presentation was 31 months, and the rate of new presentations increased five-fold during the study period. Eighty-two percent presented with prior cheiloplasty, and revision was recommended for 64 percent of them. Thirty-seven percent of patients had prior palatoplasty, of which 34 percent presented with a palatal fistula. Secondary palatoplasty/pharyngoplasty was performed more frequently for patients who underwent primary palatoplasty before adoption than after adoption (95 percent CI, 0.20 to 0.77). Overall, adoptees required secondary surgery more often than nonadoptees (49 percent versus 28 percent) regardless of where their primary surgery was performed. Changes in adoptee growth indices suggested improvements in systemic health following adoption. Conclusions: Internationally adopted children with clefts have unique treatment challenges. Children with unrepaired clefts undergo surgery late, and children with prior repairs frequently undergo revision. Compared with nonadoptees, adoptees require more revisions and have a higher fistula rate. Further detailed study is important to optimize care. (Plast. Reconstr. Surg. 134: 283e, 2014.)
C
hildren with cleft lip and/or palate who have been adopted from outside of the United States are presenting to our institution with increasing frequency. Although recent social and political changes have decreased total worldwide adoptions, patients with cleft deformities and other medical needs continue to be prioritized From the Craniofacial Center, Seattle Children’s Hospital; the Division of Plastic Surgery, Department of Surgery, University of Washington; and the Department of Plastic Surgery, University of Texas Southwestern. Received for publication November 7, 2013; accepted January 23, 2014. Presented at the 51st Annual Meeting of the Northwest Society of Plastic Surgeons, in Maui, Hawaii, February 16 through 20, 2013; and the 12th International Congress on Cleft Lip/Palate and Related Craniofacial Anomalies, in Orlando, Florida, May 5 through 10, 2013. Copyright © 2014 by the American Society of Plastic Surgeons DOI: 10.1097/PRS.0000000000000391
for adoption.1–3 The care of adoptees with clefts differs from that of children who have been managed along well-established clinical pathways that begin before birth: age of initial presentation to our center is older; previous social or physical trauma is common4; the extent and quality of previous treatment is variable; and the child-family relationship is new and evolving. We know little about how best to treat this unique group. There is scant literature on the patterns of presentation or timeline of treatment among these patients, or how they grow and develop through childhood. To date, the only published report comes from Sweden, where Hansson et al. described 25 internationally adopted patients Disclosure: The authors have no financial interest to declare in relation to the content of this article. No funding was secured for this study.
www.PRSJournal.com
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Plastic and Reconstructive Surgery • August 2014 with cleft deformities, and noted technical and social challenges to their care.5 The goal of this study was to determine the characteristics at presentation, treatment patterns, revision rates, and factors affecting revision rates among internationally adopted patients with cleft lip and/or palate.
PATIENTS AND METHODS This study was approved by our institutional review board (Seattle Children’s Hospital, no. 13968). We performed a retrospective cohort study including all children with cleft lip and/ or palate presenting to the Craniofacial Center at Seattle Children’s Hospital between 1997 and 2011. Medical records for all patients were reviewed; information pertaining to demographic factors, characteristics at presentation, and treatment (both before and after adoption) were abstracted according to a predefined rubric. We included patients born outside of the United States and Canada who were legally adopted and lived within the United States at the time of initiation of care at our center. Patients for whom adoption status was unclear and those adopted within the United States through foster care were excluded. Cleft type was determined by physical examination performed on presentation by the craniofacial pediatrician and pediatric plastic surgeon. We designated isolated cleft lip with or without cleft alveolus; cleft lip and palate; and isolated cleft palate. Cleft lip with or without cleft alveolus
and cleft lip and palate were further subclassified as unilateral or bilateral. For comparison, we abstracted information about and calculated the timing of surgical procedures and revision rates for nonadopted patients. Care at Seattle Children’s Hospital was provided in accordance with team guidelines outlined by the American Cleft Palate Association. Specialists in pediatrics, nursing, plastic surgery, otolaryngology, audiology, speech pathology, dentistry, and oral surgery provide multidisciplinary care. Records from all specialities were reviewed. Study data were collected and managed using REDCap (Research Electronic Data Capture) hosted at the University of Washington and analyses were performed using Stata version 12.1 (StataCorp, College Station, Texas).6 Univariate analysis of presentation characteristics and revision rates was performed using chi-square testing.
RESULTS Demographics Among 1841 children with cleft lip and/ or palate, 216 (12 percent) were internationally adopted. New presentations of international adoptees increased from 5 per year, to more than 25 per year from 2005 to 2010, an average five-fold increase during the study period (Fig. 1). The most common countries of origin included China (80 percent), Russia (5 percent),
Fig. 1. Incidence of internationally adopted children with cleft lip and/or palate. Consecutive patients presenting to our center from 1997 to 2011 were screened. The number of new internationally adopted patients has increased five-fold during the past 15 years. CP, isolated cleft palate; CL, cleft lip with or without cleft alveolus; CLP, cleft lip and palate.
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Volume 134, Number 2 • Adopted Children with Cleft Lip/Palate and Thailand (3 percent) (Fig. 2). Compared with U.S. adoption patterns over the same period, our population had a greater proportion of Chinese and smaller proportions of Russian and Korean adoptees.1 The male-to-female ratio was 1:1 for children with cleft lip with or without palate and 1:8 for children with isolated cleft palate. Seventy-eight percent of children had cleft lip and palate, 18 percent had isolated cleft lip with or without cleft alveolus, and 4 percent had isolated cleft palate (Fig. 3). The distribution of clefts by type remained consistent over the study period, with cleft lip and palate deformities representing greater than 50 percent of new presentations every year. The median age at adoption was 2.0 years. Most patients were first evaluated at our institution within 2 months of adoption (Fig. 4), unless they were initially cared for by another U.S. cleft team following adoption; 27 patients (13 percent) received prior surgical care at another U.S. institution before transferring to ours (data not shown). The average age at adoption remained fairly constant during the study period. Care in Country of Origin Most children with cleft lip with or without cleft alveolus had preadoption cheiloplasty (77 percent) (Fig. 5). Median age at cheiloplasty was
Fig. 3. Cleft type of international adoptees. UCL, unilateral cleft lip with or without cleft alveolus; BCL, bilateral cleft lip with or without cleft alveolus; UCLP, unilateral cleft lip and palate; BCLP, bilateral cleft lip and palate; CP, isolated cleft palate.
0.7 ± 0.6 year for unilateral cleft lip with or without cleft alveolus and 2.1 ± 1.0 years for bilateral cleft lip with or without cleft alveolus. Most children with cleft lip and palate also had preadoption cheiloplasty (85 percent); however, a minority had undergone preadoption palatoplasty (37 percent). Among children with cleft lip and palate, preadoption cheiloplasty and palatoplasty were performed at respective median ages of 1.0 and 2.2 years for unilateral cleft lip and palate and 1.4 and 2.7 years for bilateral cleft lip
Fig. 2. Country of origin of adoptees with cleft lip and/or palate presenting to our center and of overall intercountry adoptees to the United States. There were fewer than two adoptees for several countries of origin in our study group (i.e., Bolivia, Guatemala, Liberia, Philippines, Romania, and Sierra Leone). Overall intercountry adoption statistics, in thousands, were obtained from the U.S. State Department (using 1999 to 2011 data, extrapolated to the 1997 to 2011 period using a multiplier of 1.15; from U.S. Department of State. 2012 Annual Report on Intercountry Adoption. Washington, DC: Office of Children’s Issues, Bureau of Consular Affairs; 2013). Countries of origin with more than one adoptee are displayed.
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Fig. 4. Patient age at adoption and initial presentation.
and palate. Similarly, 33 percent of children with isolated cleft palate had preadoption palatoplasty, at a median age of 2.0 ± 0.1 years. A decreasing proportion of patients are undergoing surgical repair before adoption (Fig. 6). Rates of preadoption cheiloplasty dropped from 100 percent to 62 percent, and rates of preadoption palatoplasty have dropped from 62 percent
to 18 percent (an average 10 percent reduction per year for each). Rates of preadoption repair differed by sex: 91 percent of boys versus 83 percent of girls presented with preadoption cheiloplasty (p = 0.04), and 39 percent of boys versus 33 percent of girls presented with preadoption palatoplasty (p = 0.03). The rates of repair differed by country of origin.
Fig. 5. Cheiloplasty and/or palatoplasty before adoption according to cleft type. CL, cleft lip with or without cleft alveolus; CLP, cleft lip and palate; CP, isolated cleft palate.
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Fig. 6. Trends in rates of cheiloplasty and/or palatoplasty before adoption (includes years with ≥15 adoptees).
However, given the few adoptees from most countries, little can be inferred from these data. Cleft Lip: Presentation and Treatment Thirteen percent of adoptees with cleft lip (cleft lip with or without cleft alveolus or cleft lip and palate) presented with no prior repair. Of the 87 percent with prior repairs, 82 percent were performed before adoption and 5 percent were performed after adoption at another institution before transfer to our center (Table 1). Residual stigmata were common, including orbicularis muscle discontinuity (38 percent), significant nasal asymmetry (69 percent), and alar base malposition (29 percent). Lip revision was recommended for 63 percent who underwent repair before adoption and 55 percent who had undergone repair in the United States before presentation. A complete revision was recommended in most cases. Approximately 85 percent of patients in each group for whom revision was recommended chose to undergo surgery at our institution. Among patients with bilateral deformities, revision was recommended (70 percent) and carried out (53 percent) more frequently than for patients with unilateral deformities (57 percent and 42 percent, respectively). Either major or minor revision was performed in 36 percent of patients who underwent cheiloplasty at our institution, and was also more common for bilateral deformities. No association between country of
origin, side(s) of cleft, palatal involvement, sex, age at adoption, age at repair, or repair type and recommendation for revision was found. Cleft Palate: Presentation and Treatment Half (49 percent) of the adoptees with cleft palate (cleft lip and palate or isolated cleft palate) presented with no prior repair. Of the 51 percent with prior palatoplasty, 37 percent had preadoption repair and 14 percent had postadoption repair at another institution before transfer to our center (Table 2). Velopharyngeal dysfunction was noted at presentation in 48 percent of adoptees with preadoption palatoplasty and in 36 percent of adoptees with postadoption palatoplasty at another U.S. institution. Palatal fistulas were present in approximately 33 percent of patients with prior repair, either before adoption or after adoption but at another U.S. institution. Fourteen percent of patients whose primary palatoplasty was carried out at our institution developed a fistula; relative to the preadoption repair group, these patients were 70 percent less likely to develop a fistula (OR, 0.31; 95 percent CI, 0.14 to 0.69). Relative to patients with preadoption palatoplasty, palatal fistula repair was performed more frequently for patients whose palatoplasty was performed at another U.S. institution (OR, 1.39; 95 percent CI, 0.48 to 3.99) but less frequently for patients whose palatoplasty was performed at our center (OR, 0.58; 95 percent CI, 0.25 to 1.36).
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Plastic and Reconstructive Surgery • August 2014 Table 1. Cleft Lip Presentation and Treatment Patterns among Internationally Adopted Children with Cleft Lip and/or Palate Presenting to the Seattle Children’s Hospital Craniofacial Center between 1997 and 2011 Prior Repair Primary Cheiloplasty Cleft type Unilateral Bilateral Median age at cheiloplasty, yr Median age at adoption, yr Median age at presentation, yr Prior cheiloplasty at presentation Unilateral repair type Rotation advancement* Inferior triangle† Straight-line repair‡ Other Bilateral repair type Prolabial vermillion retained§ Lateral vermillion advancement║ Other Unknown Stigmata on presentation Lip length discrepancy Orbicularis discontinuity Nasal deformity Nasolabial/orolabial fistula Palatal fistula White roll discontinuity Vermillion defect/deficiency Alar base malposition Orbicularis tethered to alar base Heavy scarring Wide philtrum/prolabium Revision recommended at presentation Complete revision Minor revision¶ Patients underwent lip revision Mean age at lip revision, yr
Unrepaired
Preadoption (n = 170) (%)
Other U.S. (n = 11) (%)
SCH (n = 26)
123 (72) 47 (28) 1.1 ± 1.0 3.1 ± 2.4 3.3 ± 3.6
4 (36) 7 (64) 1.2 ± 0.5 1.2 ± 0.8 7.3 ± 6.1
22 (85) 4 (15) 1.8 ± 0.8 1.5 ± 0.7 1.6 ± 0.8
33 (59) 13 (23) 4 (7) 6 (11)
1 (50) 1 (50) 0 0
n/a n/a n/a n/a
4 (16) 19 (76) 2 (8) 95
1 (33) 2 (67) 0 6
n/a n/a n/a n/a
45 (26) 64 (38) 118 (69) 54 (32) 19 (11) 30 (18) 46 (27) 49 (29) 22 (13) 20 (12) 7 (4) 108 (64) 85 (50) 23 (14) 88 (52) 5.7 ± 3.2
2 (18) 4 (36) 8 (73) 5 (46) 2 (18) 4 (36) 5 (46) 5 (46) 2 (18) 2 (18) 2 (18) 6 (55) 4 (36) 2 (18) 5 (46) 11.5 ± 7.1
n/a n/a n/a n/a n/a n/a n/a n/a n/a n/a n/a n/a n/a n/a 9 (35) 5.7 ± 3.4
n/a, not applicable. *Millard, Mohler, and Noordhoff techniques. †Tennison-Randall techniques. ‡Nakajima and Pfeiffer techniques. §Manchester technique. ║Millard and Mulliken techniques. ¶Includes local skin flap or Z-plasty, or correction of white roll, vermillion, or mucosa.
Surgery for velopharyngeal dysfunction (e.g., secondary palatal lengthening, sphincter pharyngoplasty) was performed less often for those who underwent palatoplasty at other U.S. hospitals (OR, 0.79; 95 percent CI, 0.31 to 2.04) and at our center (OR, 0.39; 95 percent CI, 0.20 to 0.77) relative to those who underwent palatoplasty before adoption. The mean age at palatoplasty for adoptees treated at our center was the same as for those adoptees who had preadoption repair (2.4 years). Patterns among Adopted and Nonadopted Children During the study period, 1808 patients with cleft diagnoses underwent surgery at our center. Adopted children represented 10 percent of 399 patients with cleft lip with or without cleft alveolus,
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25 percent of 660 patients with cleft lip and palate, and 2 percent of 533 patients with isolated cleft palate. The overall revision rate for adoptees was higher (49 percent) than for nonadoptees (28 percent), and this was true regardless of whether they had previously undergone primary cheiloplasty/palatoplasty at our center or at another institution (Fig. 7). Given these differences, we performed a detailed review of nonadoptees who underwent palatoplasty at our institution in 2007 (the peak and median year for adoptees) to serve as a comparison group. Nonadoptees had lower fistula and revision rates than adoptees that underwent palatoplasty at our institution or at another institution (Table 2). Mean age at both primary cheiloplasty and palatoplasty was more than 1 year older for
Volume 134, Number 2 • Adopted Children with Cleft Lip/Palate Table 2. Cleft Palate Presentation and Treatment Patterns among Internationally Adopted Children with Cleft Lip and/or Palate Presenting to the Seattle Children’s Hospital Craniofacial Center between 1997 and 2011 Adoptees Unrepaired
Preadoption (n = 64) (%)
Other U.S. (n = 25) (%)
SCH (n = 86) (%)
SCH (n = 45) (%)
61 (95) 3 (5) 4.2 ± 2.6 2.4 ± 2.0 4.6 ± 2.6
25 (100) 0 (0) 1.7 ± 0.9 2.0 ± 0.9 7.0 ± 4.8
80 (93) 6 (7) 1.9 ± 1.1 2.4 ± 1.1 2.1 ± 1.1
20 (44) 25 (56) n/a 1.0 ± 0.1 n/a
31 (48) 22 (34) 33 (52) 14 (22)
9 (36) 8 (32) 11 (44) 7 (28)
n/a 12 (14)* 25 (31)* 12 (15)
n/a 3 (7)* 9 (20)* 3 (7)
25 (39) 6 (9) 2 (3) 6.3 ± 3.2 4.4 ± 3.0
6 (24) 5 (20) 0 (0) 7.2 ± 4.8 5.2 ± 4.4
13 (16) 9 (11) 3 (4) 4.9 ± 2.4 4.6 ± 3.5
4 (9) 2 (4) 0 (0) 4.2 ± 1.3 5.5 ± 1.9
Prior Repair Primary Palatoplasty Cleft type CLP iCP Median age at adoption, yr Median age at palate repair, yr Median age at presentation, yr Presentation characteristics║ Velopharyngeal insufficiency Palatal fistula† Secondary palatal surgery‡ Palatal fistula repair§ Other palatal revision surgery Furlow/secondary palatoplasty Sphincter pharyngoplasty Other Mean age at secondary palatal surgery, yr Mean follow-up from initial presentation, yr
Nonadoptees Palatoplasty in 2009
CLP, cleft lip and palate; iCP, isolated cleft palate; SCH, Seattle Children’s Hospital; n/a, not applicable. *p< 0.05 relative to the rate for those with preadoption repair. †OR, 0.90 (95% CI, 0.34 to 2.41) for other U.S. hospitals; OR, 0.31 (0.14 to 0.69) for SCH; and OR, 0.14 (95% CI, 0.04 to 0.49) for nonadoptees. ‡OR, 0.74 (95% CI, 0.29 to 1.87) for other U.S. hospitals; OR, 0.39 (95% CI, 0.20 to 0.76) for SCH; and OR, 0.23 (95% CI, 0.10 to 0.57) for nonadoptees. §OR, 1.39 (95% CI, 0.48 to 3.99) for other U.S. hospitals; OR, 0.58 (95% CI, 0.25 to 1.36) for SCH; and OR, 0.49 (95% CI, 0.13 to 1.87) for nonadoptees. ║For initially unrepaired patients, palatal fistulas are reported for the postoperative study period.
adoptees (Fig. 8). Age at palate revision also differed (mean age, 5.9 years for adoptees versus 2.9 years for nonadoptees). However, the age difference was negligible for alveolar bone grafting. Growth Patterns Age-expected percentiles for head circumference, height, and weight at presentation and at last follow-up are illustrated in Figure 9. Among adoptees who had undergone postadoption palatoplasty at another U.S. institution, there was little to no change. Adoptees who had undergone preadoption repair and presented immediately after adoption showed moderate improvements in growth measures (8, 11, and 8 percent change for head circumference, height, and weight, respectively). In adoptees who presented with unrepaired cleft palate and underwent postadoption palatoplasty at our center, the initial height and weight were lowest and improvements in all growth indices were the greatest (12, 13, and 19 percent for head circumference, height, and weight, respectively). Speech, Dental, and Otolaryngologic Patterns On initial speech and language pathologic assessment, 50 children (23 percent) were diagnosed with suspected velopharyngeal dysfunction.
Fifteen patients (7 percent) presented with articulation errors, and 36 patients (16 percent) presented with language acquisition delay. One hundred fifty-one children (70 percent) received an initial dental assessment. Of these, 39 (18 percent) had dental caries and 44 (20 percent) had a collapsed arch with crossbite. All patients with cleft palate were assessed by an otolaryngologist; 46 (26 percent) had a history of ear infections, 12 (7 percent) had had previous tympanostomy tubes placed, and 43 (25 percent) were found to have conductive hearing loss. Myringotomy tubes were indicated for 114 presenting patients (65 percent) with palatal clefting; this proportion was higher in the later years of the study period.
DISCUSSION Care of adoptees with cleft lip and/or palate differs from that of nonadopted children who have been managed since infancy. International adoptees arrive to a new country, with a new language and a new family, but also have surgical needs that have not, or only partially, been met. Despite major ongoing changes to global adoption patterns, we anticipate that the volume of adoptions
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Fig. 7. Rates of secondary surgery for adoptees and nonadoptees. SCH, Seattle Children’s Hospital.
of children with medical needs, including patients with clefts, will remain relatively stable. Presentation Our craniofacial center experienced a focal expansion of internationally adopted children with clefts approximately 10 years ago, and this population constitutes approximately 15 percent of our current cleft patient volume. The majority of adoptees at our institution are from China, and this may be because of Seattle’s location on the Pacific Rim.
Because the distribution of cleft types may vary by ethnicity, we compared our predominantly Chinese patients to a recently published distribution of cleft anomalies in China.7 A higher ratio of children with cleft lip and palate than with isolated cleft palate presented to our center than would be expected. This may reflect greater willingness to give up children with greater medical needs to families in resource-rich countries through adoption. The low incidence of adoptees with isolated cleft palate may reflect lower detection, higher
Fig. 8. Age at surgery for adoptees and nonadoptees. CL, cleft lip with or without cleft alveolus; CLP, cleft lip and palate; CP, isolated cleft palate.
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Fig. 9. Head circumference, height, and weight (as mean age-expected percentiles) among adoptees on presentation and at last follow-up. Patients are grouped according to where their palatoplasty was performed. Patients who underwent palatoplasty at another U.S. institution presented on average 5 years after adoption. Patients who underwent palatoplasty before adoption or had unrepaired cleft palate presented soon after adoption (0.4 and 0.2 years) are included. SCH, Seattle Children’s Hospital.
mortality secondary to poor feeding, or lesser willingness to give up children who do not have external facial differences. We recorded a high incidence of female adoptees: for cleft lip with or without palate, the femaleto-male ratio was 1:1, compared with the expected ratio of 1:2. For isolated cleft palate, the femaleto-male ratio was 8:1, compared with the expected ratio of 2:1.7 This could reflect a higher likelihood of giving up a girl for adoption,8 and may increase for a more visible defect. We also found that boys were significantly more likely than girls to undergo cheiloplasty or palatoplasty before adoption. Although social scientists have identified gender-preferential distribution of resources in some of the countries from which our patients originate,9 we do not have sufficient insight into the reasons for this disparity. Surgical Care Most adoptees underwent cheiloplasty before adoption, but few presented with previous palatoplasty. The reasons for this may include the timing of the adoption process, an underappreciation of
the importance of palatoplasty, a greater stigma associated with a highly visible cleft lip with or without cleft alveolus, or reluctance to proceed with a procedure associated with greater risks. We noted that the rates of both cheiloplasty and palatoplasty before adoption decreased in spite of a fairly constant age of adoption during the study. The reasons for this trend are unclear, but we speculate that rising rates of international adoption have resulted in recognition that surgical care can be deferred to a receiving team in a resource-rich country. We found that the mean age of children without prior repairs was younger than of those with prior repairs; it may be that surgery in the country of origin is deferred if an international adoption is pending. The majority of adoptees presenting to our institution with preadoption cheiloplasty required revision (64 percent), and we found a high incidence of fistulas (33 percent) and suspected velopharyngeal dysfunction (48 percent) in adoptees who underwent preadoption palatoplasty. Although the quality of previous care was sometimes excellent, in some instances, previous
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Plastic and Reconstructive Surgery • August 2014 surgical scarring precluded successful correction of residual deformities, required extensive revision, or limited results that could be achieved at the next stage of treatment. The odds that an adoptee required secondary palatal surgery was significantly greater among children who had preadoption palatoplasty relative to those who underwent postadoption palatoplasty. Better nutritional status and perioperative care following adoption may improve wound healing. However, specific inferences regarding care in the country of origin cannot be made, given that adoptees come from multiple countries, each with varying clinical practices, providers, and adoption policies. Although consensus holds that palatoplasty by 12 months optimizes speech outcomes,10 it is unclear what the relative impact of delayed palatoplasty is at older ages (i.e., 2 years) or for children facing transition to a foreign language. When consulted in specific cases where an adoption is confirmed, we sometimes consider deferring surgical treatment until arrival if the resulting delay to surgery is minimal. This provides opportunities for continuity of surgical care and for a permanent family to support the adoptee as he or she undergoes surgery. Ultimately, in our study, the mean age of palatoplasty was the same whether it was performed before or after adoption (mean, 2.4 years). In the case of unrepaired cleft lip and palate, some surgeons argue that the palate should be repaired first, given that delay may impact speech development.11 However, there are no definitive data to support this view. Other surgeons suggest simultaneous cheiloplasty and palatoplasty; however, this is technically challenging and may involve greater risks.12–14 We generally start with cleft lip repair and perform cleft palate repair within 3 months. The relative impact of this delay for adoptees is unclear, and the relative merits of the various approaches deserve further study in this population. Compared with nonadoptees at our center, fistula and surgical revision rates are higher in adoptees regardless of whether their primary repair was performed at our institution or at another. Many factors may contribute to these less favorable results, including late age at initial repair, malnutrition, and ethnic variations. We found higher revision rates for adoptees that underwent primary repair at other U.S. institutions; however, this may be attributable to referral bias, as a substantial proportion of these children transferred care because of complications or previous unfavorable results.
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Growth/Nutrition and Interdisciplinary Care We looked at trends in growth indices as a way of inferring changes in nutritional status (Fig. 9 and Table 2). Adopted children who underwent palatoplasty at another U.S. institution presented to our center on average 5 years after adoption. As expected, we measured little change in their growth percentiles because their period of catchup growth took place before arriving to our center. In contrast, children who either had preadoption repair or unrepaired cleft palate presented to our center soon after adoption (average, 0.4 and 0.2 years after adoption, respectively). We found improvements in all growth percentiles for both groups, suggesting improvements in nutritional status.15,16 Given the impact of nutritional status on wound healing, consideration of a preoperative catch-up growth period may be warranted. Additional findings on presentation included high rates of dental and otologic disease. These underscore the importance of interdisciplinary expertise of craniofacial centers in the treatment of this population.17 Speech and Social Concerns Given the implications of late palatoplasty on speech, providers and families may have an urgency to proceed with surgery but may also be reluctant to do so given the child’s vulnerable circumstances following adoption. The surgical experience for families who have recently adopted a child deserves further study to identify the factors that might predict best outcomes (including speech) for this unique population.
CONCLUSIONS Internationally adopted children with cleft lip and/or palate constitute a substantial portion of cleft patients at our institution and likely in the United States. Most of these children have undergone cleft lip repair but only one-third have undergone palate repair. Treatment of these children varies greatly from our normal protocol, as the age at primary surgery is often delayed and the revision rate is high. Increases in growth percentiles following adoption suggest improvements in systemic status that may improve surgical success. The need for secondary surgery and the rate of palatal fistula were lower among adoptees who underwent primary repair at our center than among those who underwent repair before presentation. Regardless of where the primary repairs were performed, adoptees had a higher rate of fistulae and secondary surgery compared
Volume 134, Number 2 • Adopted Children with Cleft Lip/Palate with nonadoptees. Surgical revisions help adoptees to converge with their peers who have been treated in cleft centers since infancy, and their repair timeline converges by the time of alveolar bone grafting. Raymond Tse, M.D. Division of Craniofacial and Plastic Surgery Seattle Children’s Hospital M/S OB.9.527 4800 Sand Point Way NE Seattle, Wash. 98105 [email protected]
ACKNOWLEDGMENT
The authors thank Maren Shipe for her assistance in gathering further data following peer review. REFERENCES 1. U.S. Department of State. 2012 Annual Report on Intercountry Adoption. Washington, DC: Office of Children’s Issues, Bureau of Consular Affairs; 2013. 2. Webley K. The baby deficit. Time 2013;181:34–39. 3. Johnson DE. International adoption: What is fact, what is fiction, and what is the future? Pediatr Clin North Am. 2005;52:1221–1246, v. 4. Weitzman C, Albers L. Long-term developmental, behavioral, and attachment outcomes after international adoption. Pediatr Clin North Am. 2005;52:1395–1419, viii. 5. Hansson E, Svensson H, Becker M. Adopted children with cleft lip or palate, or both, require special needs cleft surgery. J Plast Surg Hand Surg. 2012;46:75–79.
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